Topic Contents

Blood Transfusions for Sickle Cell Disease

Treatment Overview

During a blood transfusion, a person (the
recipient) receives healthy blood from another person (the donor). The donated
blood is carefully screened for diseases before it is used. Before receiving a
blood transfusion, the recipient's blood is analyzed closely (using
blood type) to make sure the donor blood is a close
match to the recipient's.

Blood is transfused into an arm vein
slowly over 1 to 4 hours (except in an emergency when blood is transfused more
quickly).

Blood transfusions aren't usually used to treat
uncomplicated
painful events or mild to moderate
anemia.

What To Expect After Treatment

Following a blood transfusion, doctors
keep a close watch on the person for any negative reactions.

Your doctor might check for iron buildup in the body
(iron overload). This can happen when a person gets many transfusions.

Why It Is Done

A blood transfusion lowers the amount
of
hemoglobin S red blood cells in the body. When there
are fewer sickled hemoglobin S cells in the bloodstream, they are less likely
to build up and block blood vessels.

Blood transfusion also
increases the number of normal red blood cells in the body, increasing the
supply of oxygen to the body.

Anemia

Some sudden complications of sickle cell
disease cause the body's red blood cell count to drop to life-threatening
levels (severe anemia). When severe, these conditions (including
splenic sequestration,
acute chest syndrome, and
aplastic crisis) can be fatal if not treated with
blood transfusions.

Surgery

After having
general anesthesia and surgery, people who have sickle
cell disease are at risk for sickling-related problems and acute chest
syndrome. Blood transfusions before surgery can prevent or treat these
complications.

Red blood cell sickling-related complications (vaso-occlusion)

Blood transfusions can treat acute chest
syndrome and leg ulcers.

Frequent blood transfusions may help
prevent strokes in children who already had a stroke or are at high risk for a
first stroke.footnote 1

How Well It Works

Blood transfusion is an effective and proven treatment for some severe complications of
sickle cell disease.footnote 2

Blood transfusions
reduce the risk of some complications of sickle cell disease and reduce symptoms of severe anemia.

Blood transfusion may help prevent a first stroke in children who have sickle cell
disease. Transfusions can help prevent a second stroke in most children. But some children
who get repeat transfusions will still have a second stroke.footnote 3

Risks

A person receiving many blood transfusions will
gradually collect too much iron in the body (iron overload). Very high levels
of iron can lead to
hemochromatosis, which can be fatal if untreated.

A person receiving repeat blood transfusions may develop
antibodies to the donor blood. This is called
alloimmunization. Alloimmunization makes repeated
transfusions more difficult. It occurs in about 1 out of 4 people who have sickle
cell disease and who get frequent transfusions.footnote 4

A transfusion reaction can result from mismatched blood type. And it may
occur immediately or days later. An acute
transfusion reaction ranges from mild (fever, chills, and rash) to severe
(shock, severe anemia, painful event, and death).

Donated blood is carefully screened for diseases before it
is used. It is very rare for a disease to be transmitted through donated blood.

What To Think About

If you have repeated blood
transfusions, you'll have routine testing for iron buildup in your body.

Though blood transfusions are an effective treatment for sickle
cell disease complications, they are only used selectively. The risks of
hemochromatosis and alloimmunization from repeat transfusions makes this
procedure more suitable for severe and high-risk conditions.

References

Citations

Goldstein LB, et al. (2010). Guidelines for the primary prevention of stroke: A guideline for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. Published online December 2, 2010 (doi: 10.1161/STR.0b013e3181fcb238). Also available online: http://stroke.ahajournals.org/content/42/2/517.full.

Goldstein LB, et al. (2010). Guidelines for the primary prevention of stroke: A guideline for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. Published online December 2, 2010 (doi: 10.1161/STR.0b013e3181fcb238). Also available online: http://stroke.ahajournals.org/content/42/2/517.full.

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